Lousa M, Sastre J L, Cancelas J A, Gobernado J M, Pardo A
Department of Neurology, Hospital Ramón y Cajal, Madrid, Spain.
Stroke. 1994 May;25(5):1071-4. doi: 10.1161/01.str.25.5.1071.
Sneddon's syndrome is a disease characterized by livedo reticularis and cerebrovascular lesions, with a hereditary transmission and unknown etiopathogenesis. A number of reports have documented a link between antiphospholipid antibodies and Sneddon's syndrome with different results. The present work was designed to sequentially study antiphospholipid antibodies in a patient with Sneddon's syndrome and her family and their potential role in thrombotic events. We used cardiolipin and a mixture of phospholipids from rabbit brain as antigen for antiphospholipid assays to determine diagnostic usefulness.
A patient with Sneddon's syndrome and 12 available family members belonging to three generations were evaluated to determine the presence of antiphospholipid antibodies (lupus anticoagulant and anticardiolipin antibodies) during vascular thrombotic events and asymptomatic periods.
Our results support a temporal relation between thrombotic events in Sneddon's syndrome and lupus anticoagulant; anticardiolipin antibodies remained invariable. Our index case patient and her father could be diagnosed as having primary antiphospholipid antibody syndrome. Aspirin was not effective in preventing thrombosis. After the onset of oral anticoagulant therapy, no recurrences were seen. The use of a mixture of phospholipids as antigen could present some advantages in serological studies performed in antiphospholipid syndromes.
斯内登综合征是一种以网状青斑和脑血管病变为特征的疾病，具有遗传传递性且病因不明。许多报告记录了抗磷脂抗体与斯内登综合征之间的联系，但结果各异。本研究旨在对一名斯内登综合征患者及其家族中的抗磷脂抗体进行序贯研究，以及它们在血栓形成事件中的潜在作用。我们使用心磷脂和兔脑磷脂混合物作为抗磷脂检测的抗原，以确定其诊断价值。
对一名患有斯内登综合征的患者以及属于三代的12名在世家庭成员进行评估，以确定在血管血栓形成事件和无症状期抗磷脂抗体（狼疮抗凝物和抗心磷脂抗体）的存在情况。
我们的结果支持斯内登综合征血栓形成事件与狼疮抗凝物之间存在时间关系；抗心磷脂抗体保持不变。我们的索引病例患者及其父亲可被诊断为患有原发性抗磷脂抗体综合征。阿司匹林在预防血栓形成方面无效。开始口服抗凝治疗后，未见复发。在抗磷脂综合征的血清学研究中，使用磷脂混合物作为抗原可能具有一些优势。
